Two case reports of rare diseases occurring in rare parts: Splenic vein solitary fibrous tumor and Liver solitary fibrous tumor

2020 ◽  
Author(s):  
Wenjing Wang ◽  
Banghe Bao ◽  
Anbin Hu ◽  
Xiaofeng Zhu ◽  
Qing Chen
Keyword(s):  
Liver Transplantation ◽  
Surgical Resection ◽  
Orthotopic Liver Transplantation ◽  
Solitary Fibrous Tumor ◽  
Primary Tumor ◽  
Splenic Vein ◽  
Tumor Site ◽  
Primary Tumor Site ◽  
Fibrous Tumor

Abstract Background Solitary fibrous tumor (SFT) is a rare soft tissue tumor originating from mesenchyme. Two cases of SFT we report right now occurred in the splenic vein and liver respectively, this primary splenic vein SFT may be the first report case, and also the first report of liver recurrence SFT cured by orthotopic liver transplantation (OLT). Case presentation One case was a 37-year-old female patient whose primary tumor site was located in the splenic vein, which resulted in splenomegaly and hypersplenism; its recurrence again and again after surgical resection and eventually transferred to the liver, during 10 years of follow-up, 4 operations were performed, and he is in a good condition right now. The second case was a 54-year-old male patient whose primary tumor site was located in the liver, spleen and left side of the chest wall; however, he had no uncomfortable symptoms. Surgeons performed two operations to remove these tumors, totally. 6 years later, SFT recurrence in the liver, and given that the tumor was so large that it could not be completely surgical resected, we chose orthotopic liver transplantation (OLT), and no tumor recurrence during 12-month follow-up. Conclusion The reports of these two cases of SFT are very rare, especially the splenic vein SFT, which expand the understanding of SFT. The main treatment of SFT is still surgical resection, right now, and liver transplantation may be a new option treatment for the huge liver SFT.

scholarly journals Solitary fibrous tumor of the pleura: A rare mesenchymal tumor presented with hypoglycemia

2021 ◽  
Vol 5 (1) ◽  
pp. 22-25
Author(s):  
Yasser Ali Kamal ◽  
Keyword(s):  
Chest Pain ◽  
Surgical Resection ◽  
Clinical Features ◽  
Solitary Fibrous Tumor ◽  
Primary Tumor ◽  
Clinical Laboratory ◽  
Mesenchymal Cells ◽  
Radiological Features ◽  
Complete Surgical Resection ◽  
Fibrous Tumor

Solitary fibrous tumor (SFT) is a relatively rare primary tumor of the pleura, originating from the mesenchymal cells. The tumor has a non-specific clinical, laboratory, and radiological features. Patients may be asymptomatic or have non-specific clinical features including chest pain, dyspnea, cough, and paraneoplastic symptoms. Complete surgical resection is indicated in all cases to avoid recurrence of the tumor. We described a 49-yearold woman who presented with unexplained right chest pain and repeated attacks of hypoglycemia, associated with pleural SFT. Keywords: Pleura; thoracic oncology; solitary fibrous tumor; paraneoplastic hypoglycemia; Doege-Potter syndrome

scholarly journals Primary Tumor Site Affects Survival in Patients with Gastroenteropancreatic and Neuroendocrine Liver Metastases

2019 ◽  
Vol 2019 ◽  
pp. 1-7 ◽  
Author(s):  
John F. Tierney ◽  
Jennifer Poirier ◽  
Sitaram Chivukula ◽  
Sam G. Pappas ◽  
Martin Hertl ◽  
...  
Keyword(s):  
Liver Metastases ◽  
Surgical Resection ◽  
Primary Tumor ◽  
Multivariable Analysis ◽  
Cox Proportional Hazards ◽  
Tumor Site ◽  
Gastroenteropancreatic Neuroendocrine Tumors ◽  
Primary Tumor Site ◽  
Metastatic Site ◽  
Extrahepatic Metastases

Background. Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are commonly present with metastatic disease, and the liver is the most frequent metastatic site. Herein, we studied whether primary tumor site affects survival in patients with GEP-NETs and liver metastases (NELM). As a secondary endpoint, we studied whether extrahepatic disease and surgical resection impact survival in this patient population. Methods. Patients with NELM diagnosed from 2006 to 2014 were identified from the National Cancer Database. Kaplan-Meier curves and nested Cox proportional hazards were used to assess variables associated with survival. Results. 2947 patients with well- or moderately differentiated GEP-NETs and NELM met the inclusion criteria for this study. Patients with small bowel NETs survived the longest of all GEP-NETs with NELM (median not reached). Rectal and gastric NETs with NELM had the shortest survival (median 31 months). Patients with extrahepatic metastases who underwent any operation survived longer than those managed nonoperatively (median survival 38.7 months vs. 18.6 months, p=0.01). On multivariable analysis, operations on the primary tumor and distant metastatic site (HR 0.23-0.43 vs. no surgery), treatment at an academic/research hospital, Charlson comorbidity index of 0, no extrahepatic metastases, and younger age were associated with prolonged survival (p<0.01). Conclusions. Primary tumor site affects survival in patients with GEP-NETs and NELM. Surgical resection seems beneficial for all GEP-NETs with NELM, even in the presence of extrahepatic metastases.

scholarly journals Do Metastatic Kidney Cancer Patients Benefit From Cytoreductive Nephrectomy? A Real-World Retrospective Study From the SEER Database

2021 ◽  
Vol 8 ◽  
Author(s):  
Cheng Li ◽  
Ruiliang Wang ◽  
Wenchao Ma ◽  
Shenghua Liu ◽  
Xudong Yao
Keyword(s):  
Surgical Resection ◽  
Kidney Cancer ◽  
Real World ◽  
Primary Tumor ◽  
Target Therapy ◽  
Tumor Site ◽  
Seer Database ◽  
Cytoreductive Nephrectomy ◽  
Cancer Specific Survival ◽  
Primary Tumor Site

Introduction: The benefit of cytoreductive nephrectomy (CN) for metastatic kidney cancer has been challenged recently. The study aimed to evaluate the prognostic roles of surgical resection of primary tumor site for metastatic kidney cancer under a real-world setting.Methods: The Surveillance, Epidemiology, and End Results (SEER) database (2010–2015) and the overall survival (OS) and cancer-specific survival (CSS) were evaluated using the Cox proportional hazards regression model. One-to-one matching using the propensity score was used to estimate and compare the survival rates.Results: The SEER data contain records of 8,932 patients from 2010 to 2015. The data showed that 61.7% of the patients underwent CN while 38.2% did not receive any surgery. The median survival month for a patient without surgery was 4 months and for a patient with surgery was 19 months. The multivariate analysis showed that surgical resection of the primary tumor site was an independent favorable predictor for both OS and CSS (all p &lt; 0.001) in the original and the matching cohort.Conclusions: In the era of target therapy, CN might still be a vital method to treat metastatic kidney cancer.

Bone scintigraphy in nonsurgically treated Ewing's sarcoma at diagnosis and follow-up: Prognostic information of the primary tumor site

1994 ◽  
Vol 22 (4) ◽  
pp. 236-239 ◽  
Author(s):  
Elise A. L. Hugenholtz ◽  
Do A. Piers ◽  
Willem A. Kamps ◽  
Ian J. Cohen ◽  
Chedva Lerman ◽  
...  
Keyword(s):  
Bone Scintigraphy ◽  
Primary Tumor ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Tumor Site ◽  
Prognostic Information ◽  
Primary Tumor Site

Pazopanib-associated secondary adrenal insufficiency in a patient with malignant solitary fibrous tumor

2021 ◽  
pp. 107815522110160
Author(s):  
Muhammed Muhiddin Er ◽  
Murat Araz ◽  
Meryem Karabacak ◽  
Muzaffer Uğraklı ◽  
Melek Karakurt Eryılmaz ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Solitary Fibrous Tumor ◽  
Single Case ◽  
Soft Tissue Sarcomas ◽  
Pituitary Hormones ◽  
Secondary Adrenal Insufficiency ◽  
Primary Adrenal Insufficiency ◽  
Hypoglycemic Attack ◽  
Fibrous Tumor

Introduction Pazopanib is an agent that is being successfully used in soft tissue sarcomas. Some endocrine side effects may develop during pazopanib treatment. Here, we presented a case diagnosed with secondary adrenal insufficiency while being investigated for etiology of hypoglycemia which developed after pazopanib. Case report A 69-year-old male patient was operated in June 2019 due to a lung mass 26 × 18 × 10 cm in size. Pathological diagnosis revealed a solitary fibrous tumor with malignant behavior. The patient received three lines of chemotherapy. After pazopanib treatment, a hypoglycemic attack was reported. Management and outcome: Blood cortisol and ACTH (Adrenocorticotropic hormone) levels were not increased at the time of the hypoglycemic attack, and levels of other pituitary hormones were found to be normal. Electrolyte levels were in normal range. Since the counteracting hormone did not reach a sufficient level, it was considered secondary adrenal insufficiency. Hypoglycemic attacks did not occur during follow-up while taking steroid therapy and pazopanib. Discussion A single case of primary adrenal insufficiency has been reported in the literature. We here present a case who developed hypoglycemia after pazopanib and was diagnosed with drug-associated secondary adrenal insufficiency. When hypoglycemia develops during pazopanib treatment, we must be aware of adrenal insufficiency.

Comparison of treatment regimens for pediatric lymphoblastic non-Hodgkin's lymphoma: a Childrens Cancer Group study.

1995 ◽  
Vol 13 (6) ◽  
pp. 1368-1376 ◽  
Author(s):  
D G Tubergen ◽  
M D Krailo ◽  
A T Meadows ◽  
J Rosenstock ◽  
M Kadin ◽  
...  
Keyword(s):  
Primary Tumor ◽  
Hodgkin’S Lymphoma ◽  
Myelogenous Leukemia ◽  
Hodgkin's Lymphoma ◽  
Tumor Site ◽  
Primary Tumor Site ◽  
Non Hodgkin’S Lymphoma ◽  
Non Hodgkin's Lymphoma ◽  
Localized Disease ◽  
Extent Of Disease

PURPOSE Patients with lymphoblastic non-Hodgkin's lymphoma (LB NHL) were randomized to treatment with either modified LSA2L2 or ADCOMP, which added daunorubicin (DAUN) and asparaginase (L-ASP) to the methotrexate (MTX), cyclophosphamide (CYT), vincristine (VCR), and prednisone (PRED) (COMP) regimen, in a clinical trial to determine the relative effectiveness and toxicity of the two regimens. PATIENTS AND METHODS Patients with LB NHL were eligible for this randomized study if they were less than 22 years of age at diagnosis and had < or = 25% blasts in the bone marrow. Of 307 patients registered, 281 were fully eligible and assessable. Patients were stratified by extent of disease at diagnosis. RESULTS The 5-year event-free survival (EFS) rate for patients with localized disease was 84%, and for patients with disseminated disease, 67%. There were four relapses in 28 patients with localized disease. Two hundred six patients had mediastinal primary tumors and despite local radiation, 34 of 63 failures in these patients involved the primary tumor site with or without other involvement. After adjusting for extent of disease at diagnosis, the regimens did not differ significantly with respect to risk for adverse events. The acute toxicity was primarily neutropenia and thrombocytopenia, with greater initial toxicity in patients on the LSA2L2 regimen. Three patients developed acute myelogenous leukemia. CONCLUSION Long-term EFS in children with LB NHL can be achieved in the majority of patients. Disease progression, which includes recurrence at the primary tumor site, is a major cause of treatment failure in patients with mediastinal presentations. Addition of DAUN and L-ASP to the COMP regimen does not produce a more effective treatment than LSA2L2.

A phase II/III randomized double-blind study of octreotide acetate LAR with axitinib versus octreotide acetate LAR with placebo in patients with advanced G1-G2 NETs of non-pancreatic origin (AXINET trial-GETNE-1107).

2021 ◽  
Vol 39 (3_suppl) ◽  
pp. 360-360
Author(s):  
Rocio Garcia-Carbonero ◽  
Marta Benavent ◽  
Paula Jiménez Fonseca ◽  
Daniel Castellano ◽  
Teresa Alonso ◽  
...  
Keyword(s):  
Disease Progression ◽  
Phase Ii ◽  
Primary Tumor ◽  
Phase Iii ◽  
Study Entry ◽  
Tumor Site ◽  
Double Blind ◽  
Primary Tumor Site ◽  
Octreotide Lar ◽  
Octreotide Acetate

360 Background: Angiogenesis plays an important role in NET development and progression. Axitinib is a potent and selective VEGFR-1,2,3 inhibitor, with proven activity against several vascular-dependent solid tumors. The aim of this randomized, double-blind phase II/III study was to assess the efficacy of axitinib in patients with advanced G1-2 extra-pancreatic NETs. Methods: Eligible pts were randomized (1:1) to receive octreotide LAR (30 mg IM q4w) with axitinib (5 mg BID) or placebo BID until disease progression or unacceptable toxicity. Pteswere stratified by time from diagnosis to study entry ( > or < 12m), primary tumor site (GI tract vs non-GI) and Ki-67 index (< 5% vs > 5%). Prior therapy with SSA, IFN and up to 2 lines of systemic treatment was allowed, but not prior VEGF- or VEGFR-targeted drugs. Clinical and/or radiological disease progression within 12 months prior to study entry was required. The primary endpoint was progression-free survival (PFS). Secondary endpoints included overall survival (OS), time to progression, overall response rate (ORR), duration of response, biochemical response and safety. Results: 256 pts were randomized (106 in the Phase II part, and 150 additional pts in the Phase III part), 126 to axitinib and 130 to placebo. The main characteristics of the study population were: median age 61 years (range: 21-85), 52% male, PS 0-1 (64-35%), G1-2 (29%-71%), primary tumor site GI (40%)-Lung (17%)-Other (32%). Prior therapies included: SSA (46%), everolimus (13%), chemotherapy (13%), TACE (5%) and PRRT (2%). ORR was significantly higher in axitinib- vs placebo-treated patients (17.5% vs 3.8%, p = 0.0004). PFS per investigator assessment also favored axitinib vs placebo-treated patients, although the difference did not reach statistical significance (median PFS 17.2 vs 12.3 months, respectively, HR 0.816, p = 0.169). Grade 3-4 treatment-related AEs occurred more frequently in the axitinib vs placebo arm (52% vs 13.8%), and included hypertension (21% vs 6 %), cardiac disorders (3.2% vs 0.7%), diarrhoea (13% vs 1.5 %), asthenia (9% vs 3%) and nausea&vomiting (2% vs 0.7%). There were 3 treatment-related deaths, 1 in the axitinib arm (cardiac failure) and 2 in the placebo arm (myocardial infarction and hepatorenal syndrome). Conclusions: Although the study failed to demonstrate a significant PFS benefit per investigator assessment, axitinib in combination with octreotide LAR demonstrated activity and had a tolerable safety profile in patients with advanced G1-2 extra-pancreatic NETs. Data base cleaning and central blinded radiological PFS assessment are currently ongoing. Clinical trial information: NCT01744249.

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